In this case report, an 89-year-old woman presented with a subcutaneous tumour in the orbital region. Fine needle aspiration showed no signs of malignancy. Following progressive tumour growth and cutaneous manifestation, an incisional biopsy was taken, which showed squamous cell carcinoma. The tumour was excised, and the patho-logical report showed necrobiotic xanthogranuloma being a rare histiocytic disease characterised by slow growth of nodules and plaques. Because it is rare, it can pose a diagnostic challenge, which is why, this report is an attempt to shed light on the disease and to aid its diagnosis.